-
American Journal of Clinical Dermatology May 2021Erythema nodosum is the most common form of panniculitis and is characterized by tender erythematous nodules mainly in the lower limbs on the pretibial area. The exact... (Review)
Review
Erythema nodosum is the most common form of panniculitis and is characterized by tender erythematous nodules mainly in the lower limbs on the pretibial area. The exact cause of erythema nodosum is unknown, although it appears to be a hypersensitivity response to a variety of antigenic stimuli. Although the etiology is mostly idiopathic, ruling out an underlying disease is imperative before diagnosing primary erythema nodosum. Erythema nodosum can be the first sign of a systemic disease that is triggered by a large group of processes, such as infections, inflammatory diseases, neoplasia, and/or drugs. The most common identifiable causes are streptococcal infections, primary tuberculosis, sarcoidosis, Behçet disease, inflammatory bowel disease, drugs, and pregnancy. We propose a diagnostic algorithm to optimize the initial work-up, hence initiating prompt and accurate management of the underlying disease. The algorithm includes an initial assessment of core symptoms, diagnostic work-up, differential diagnosis, and recommended therapies. Several treatment options for the erythema nodosum lesions have been previously reported; nevertheless, these options treat the symptoms, but not the triggering cause. Making an accurate diagnosis will allow the physician to treat the underlying cause and determine an optimal therapeutic strategy.
Topics: Anti-Inflammatory Agents; Diagnosis, Differential; Drug Therapy, Combination; Erythema Nodosum; Humans; Incidence; Skin; Treatment Outcome
PubMed: 33683567
DOI: 10.1007/s40257-021-00592-w -
American Journal of Clinical Dermatology May 2022Sweet syndrome (SS), or acute febrile neutrophilic dermatosis, is an inflammatory, non-infectious skin reaction characterized clinically by tender, erythematous... (Review)
Review
Sweet syndrome (SS), or acute febrile neutrophilic dermatosis, is an inflammatory, non-infectious skin reaction characterized clinically by tender, erythematous papules/plaques/pustules/nodules commonly appearing on the upper limbs, trunk, and head and neck; histologically, SS is characterized by dense neutrophilic infiltrate in the dermis. SS is accompanied by fever; an elevation of inflammatory markers (e.g., erythrocyte sedimentation rate, C reactive protein) in serum may also be observed. Although most cases of SS are idiopathic, SS also occurs in the setting of malignancy or following administration of an associated drug. SS has also been reported in association with pregnancy and a burgeoning list of infectious (most commonly upper respiratory tract infections) and inflammatory diseases; likewise, the litany of possible iatrogenic triggers has also grown. Over the past several years, a wider spectrum of SS presentation has been realized, with several reports highlighting novel clinical and histological variants. Corticosteroids continue to be efficacious first-line therapy for the majority of patients with SS, although novel steroid-sparing agents have been recently added to the therapeutic armamentarium against refractory SS. New mechanisms of SS induction have also been recognized, although the precise etiology of SS still remains elusive. Here, we catalogue the various clinical and histological presentations of SS, summarize recently reported disease associations and iatrogenic triggers, and review treatment options. We also attempt to frame the findings of this review in the context of established and emerging paradigms of SS pathogenesis.
Topics: Adrenal Cortex Hormones; Humans; Iatrogenic Disease; Neck; Skin; Sweet Syndrome
PubMed: 35157247
DOI: 10.1007/s40257-022-00673-4 -
The Chinese Journal of Physiology 2021Hidradenitis suppurativa is a cutaneous chronic inflammatory disease that is estimated to affect about 1% of the population and caused pain, malodorous discharge,... (Review)
Review
Hidradenitis suppurativa is a cutaneous chronic inflammatory disease that is estimated to affect about 1% of the population and caused pain, malodorous discharge, disfigurement, and poor quality of life with psychosocial problems. The typical features are recurrent painful nodules, abscesses, and sinus tracts on the axillae, groins, gluteal areas, and anogenital regions since postpuberty. Smoking and obesity are two major triggering factors of hidradenitis suppurativa. Women are prone to have hidradenitis suppurativa than men in Western countries, but the male-to-female ratio is reversed in oriental countries. The disease severity can be affected by menstruation, pregnancy, and menopause. Furthermore, the phenotypes are different among men and women with hidradenitis suppurativa. Men are prone to have buttock involvement while women are prone to have axillary, groins, and submammary lesions. This review introduces the skin appendages and pathophysiology of hidradenitis suppurativa and then focuses on the sex difference and the effects of sex hormones on hidradenitis suppurativa and current hormone-associated treatments.
Topics: Female; Gonadal Steroid Hormones; Hidradenitis Suppurativa; Humans; Inflammation; Male; Quality of Life; Skin
PubMed: 34975118
DOI: 10.4103/cjp.cjp_67_21 -
American Journal of Clinical Dermatology Mar 2022Hidradenitis suppurativa (HS) is a severe chronic relapsing inflammatory disorder of the hair follicle unit that can cause painful abscesses, nodules, tunnels, and... (Review)
Review
Hidradenitis suppurativa (HS) is a severe chronic relapsing inflammatory disorder of the hair follicle unit that can cause painful abscesses, nodules, tunnels, and tracts in intertriginous parts of the body. The disease can often result in disfigurement and adversely impact patient quality of life. The management of HS has expanded significantly over the past decade to include multiple modalities, including topical therapies, systemic therapies (non-biologics and biologics), surgical therapies, lifestyle changes, and management of comorbidities. Management can often be clinically challenging and may involve the combination of medical and surgical approaches for optimal results. The purpose of this review is to present an update on non-biologic and non-interventional modalities published in 2019-2021 in the clinical management of HS. With emerging therapies, ongoing clinical trials, and heightened awareness about HS, there is hope that new treatment options will revolutionize the management of patients suffering from HS.
Topics: Abscess; Comorbidity; Hair Follicle; Hidradenitis Suppurativa; Humans; Quality of Life
PubMed: 34990004
DOI: 10.1007/s40257-021-00667-8 -
Actas Dermo-sifiliograficas Nov 2020Juvenile xanthogranulomas (JXGs) are rare, benign lesions that belong to the large group of non-Langerhans cell histiocytoses. JXG presents with 1 or more erythematous... (Review)
Review
Juvenile xanthogranulomas (JXGs) are rare, benign lesions that belong to the large group of non-Langerhans cell histiocytoses. JXG presents with 1 or more erythematous or yellowish nodules that are usually located on the head or neck. Most JXG lesions are congenital or appear during the first year of life. Extracutaneous involvement is rare, but the literature traditionally suggests investigating the possibility of ocular compromise. JXG is mainly a clinical diagnosis, but a skin biopsy may sometimes be needed for confirmation. JXGs on the skin are self-limiting and usually do not require treatment. This review describes the clinical and therapeutic aspects of JXG, emphasizing available evidence and the diagnosis of extracutaneous involvement.
Topics: Biopsy; Histiocytosis, Non-Langerhans-Cell; Humans; Skin; Xanthogranuloma, Juvenile
PubMed: 32721389
DOI: 10.1016/j.ad.2020.07.004 -
Advances in Therapy Dec 2020Calciphylaxis is a deadly, painful disease with a 1-year mortality of up to 50%. The disease is commonly associated with patients with end-stage kidney disease (ESKD),... (Review)
Review
Calciphylaxis is a deadly, painful disease with a 1-year mortality of up to 50%. The disease is commonly associated with patients with end-stage kidney disease (ESKD), but it can manifest in non-uremic patients as well. In patients who are undergoing dialysis, the incidence of calciphylaxis can range from 0.04% to 4%. The progressive arterial calcification seen in calciphylaxis can affect multiple body organs, including the skin, brain, lungs, and muscle. In cutaneous calciphylaxis, painful and non-healing nodules, plaques, and ulcers may appear, increasing morbidity for patients. Diagnosis can be difficult, and the condition can clinically appear similar to other dermatological diseases, especially in non-uremic patients. Currently, skin biopsy with histological analysis is the most reliable method to help diagnose the condition. In certain cases, the use of medical imaging may be helpful. Treatment of pain in this condition can be difficult and should be multimodal and include wound care as well as modification of risk factors. Analgesic options include opioids, nonsteroidal anti-inflammatory drugs (NSAIDs), as well as analgesic options that are targeted for specific patients. There are currently multiple clinical trials underway that are studying targeted therapies for this condition.
Topics: Calciphylaxis; Disease Management; Disease Progression; Humans; Kidney Failure, Chronic; Renal Dialysis; Risk Factors; Skin
PubMed: 32997277
DOI: 10.1007/s12325-020-01504-w -
The Journal of Allergy and Clinical... Jan 2024Prurigo nodularis (PN) is a chronic neuroimmune skin disease characterized by bilaterally distributed pruritic hyperkeratotic nodules on extremities and trunk....
BACKGROUND
Prurigo nodularis (PN) is a chronic neuroimmune skin disease characterized by bilaterally distributed pruritic hyperkeratotic nodules on extremities and trunk. Neuroimmune dysregulation and chronic scratching are believed to both induce and maintain the characteristic lesions.
OBJECTIVES
This study sought to provide a comprehensive view of the molecular pathogenesis of PN at the single-cell level to identify and outline key pathologic processes and the cell types involved. Features that distinguish PN skin from the skin of patients with atopic dermatitis were of particular interest. We further aimed to determine the impact of the IL31RA antagonist, nemolizumab, and its specificity at the single-cell level.
METHODS
Single-cell RNA-sequencing of skin from 15 healthy donors and nonlesional and lesional skin from 6 patients each with PN and atopic dermatitis, combined with spatial-sequencing using the 10x Visium platform. Integration with bulk RNA-sequencing data from patients treated with nemolizumab.
RESULTS
This study demonstrates that PN is an inflammatory skin disease characterized by both keratinocyte proliferation and activation of profibrotic responses. This study also demonstrates that the COL11A1 fibroblast subset is a major contributor to fibrosis and is predominantly found in the papillary dermis of PN skin. Activation of fibrotic responses is the main distinguishing feature between PN and atopic dermatitis skin. This study further shows the broad effect of nemolizumab on PN cell types, with a prominent effect driving COL11A1 fibroblast and keratinocyte responses toward normal.
CONCLUSIONS
This study provides a high-resolution characterization of the cell types and cellular processes activated in PN skin, establishing PN as a chronic fibrotic inflammatory skin disease. It further demonstrates the broad effect of nemolizumab on pathological processes in PN skin.
Topics: Humans; Prurigo; Dermatitis, Atopic; Skin; Chronic Disease; RNA; Pruritus
PubMed: 37506977
DOI: 10.1016/j.jaci.2023.07.005 -
The American Journal of Tropical... Sep 2016Scabies is a common contagious cutaneous disease and usually affects the young, characterized by polymorphous lesions that may present as burrows, pruritic papules, and... (Review)
Review
Scabies is a common contagious cutaneous disease and usually affects the young, characterized by polymorphous lesions that may present as burrows, pruritic papules, and inflammatory nodules. Bullous scabies (BS) is its rather rare subtype, mimicking bullous pemphigoid. We report a 15-year-old Chinese boy presenting with 1-month history of pruritic bullae on his penile skin, showing poor response to both topical steroids and systemic antihistamines, but cured by sulfur ointment alone. No recurrence occurred in the 5 years of follow-up. We also reviewed the published cases. Up to date, 44 cases, including the present, have been reported. Of them, 30 were male and 14 were female. The age range was from 1 to 89 years old, with a median age of 70.6 years. The bullous lesions may involve the arms, legs, trunk, genitals, feet, buttocks, thighs, neck, inguinal folds, and may even be generalized. Trunk and extremities are the most common involved locations. Facial or mucosa involvement had never been reported. The histological findings present as a subepidermal split with variable inflammatory infiltrate predominantly neutrophils, and eosinophilic spongiosis, or both. Eighteen of 32 patients showed positive deposition of linear-granular IgG or complement 3 alone or in various combinations, and five of 24 patients revealed circulating IgG. All the 40 cases with therapeutic details were cured by antiscabietic remedy. BS always involves the trunk and extremities. It has a predilection for elderlies and males. The treatments for BS are similar to those of classical scabies.
Topics: Adolescent; Blister; Humans; Male; Penis; Scabies; Skin
PubMed: 27402514
DOI: 10.4269/ajtmh.16-0273 -
The Journal of Investigative Dermatology Feb 2022Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease characterized by the formation of nodules, abscesses, and fistulae at intertriginous sites. The... (Observational Study)
Observational Study
Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease characterized by the formation of nodules, abscesses, and fistulae at intertriginous sites. The skin-gut axis is an area of emerging research in inflammatory skin disease and is a potential contributory factor to the pathogenesis of HS. A total of 59 patients with HS provided fecal samples and nasal and skin swabs of affected sites for analysis. A total of 30 healthy controls provided fecal samples, and 20 healthy controls provided nasal and skin swabs. We performed bacterial 16S ribosomal RNA gene amplicon sequencing on total DNA derived from the samples. Microbiome alpha diversity was significantly lower in the fecal, skin, and nasal samples of individuals with HS, which may be secondary to disease biology or related to antibiotic usage. Ruminococcus gnavus was more abundant in the fecal microbiome of individuals with HS, which is also reported in Crohn's disease, suggesting comorbidity due to shared gut microbiota alterations. Finegoldia magna was overabundant in HS skin samples relative to that in the healthy controls. It is possible that local inflammation is driven by F. magna by promoting the formation of neutrophil extracellular traps. These alterations in both the gut and skin microbiome in HS warrant further exploration, and therapeutic strategies, including fecal microbiota transplant or bacteriotherapy, could be of benefit.
Topics: Adult; Aged; Case-Control Studies; Clostridiales; Extracellular Traps; Fecal Microbiota Transplantation; Feces; Female; Firmicutes; Gastrointestinal Microbiome; Hidradenitis Suppurativa; Humans; Male; Middle Aged; Skin; Young Adult
PubMed: 34364884
DOI: 10.1016/j.jid.2021.05.036 -
Acta Medica Indonesiana Apr 2023Diagnosis of nodular red lesions is challenging. The differential diagnosis includes dermal nevus, angioma, pyogenic granuloma, amelanotic melanoma, eccrine poroma,...
Diagnosis of nodular red lesions is challenging. The differential diagnosis includes dermal nevus, angioma, pyogenic granuloma, amelanotic melanoma, eccrine poroma, Kaposi's sarcoma, skin malignancy or metastasis. Erythema nodosum is one of the common consideration of the red skin nodules, however fully work up should be done to find the right diagnosis.A 60 years old female admitted to our hospital due to pain dark reddish skin nodules since one month. She had continuously high grade fever of 39 Celsius accompanied by arthralgia and fatigue since two months prior to admission and she lost 6 kg of weight in 2 months. On admission, physical examination revealed slight fever, pale conjunctiva, mild hepatosplenomegaly, tender dark red nodules 0.3 to 2 cm, firm edge, at her cheek, abdominal area and both lower extremities. No lymph nodes enlargement was noticed. Her laboratory test showed haemoglobin 9,1 g/dl, WBC 3,040/mL, PLT 149,000/mL, SGOT 48 U/L, SGPT 43 U/L, urea 12.5 mg/dL, creatinine 0.67 mg/dL. She was found to be non-reactive for HBsAg, HCV, and HIV antigens. Urine routine and microscopic examination was unremarkable.Her histopathology of left foot nodule biopsy revealed cutaneous lymphoma. The immunohistochemical (IHC) stain of CD45, CD20, and CD10 were positive, Ki67 were also positive with >70% tumor cells, while CD3,CD56, CD30, and Granzyme were negative. Her final diagnosed was Cutaneous Diffuse large B cell lymphoma.Primary cutaneous lymphomas of B-cells occur less frequently than primary cutaneous T-cells lymphomas. Primary extra-nodal diffuse large B-Cell lymphoma (DLBCL) can be seen in up to 40% of cases. However skin involvement is less common and in a large cohort of DLBCL cases, skin involvement at presentation was seen only in 3.3% of cases.It characterized by few lesions, in general showing nodules or infiltrations of relatively fast growth and have no itching. The diagnosis is made by the immunohistochemical findings, clinicopathological correlation, and molecular pathology. The lymphomas have different clinical behaviours despite being identical in morphological appearance. The primary lymphomas presents with local recurrence in up to 68% of the cases and with rare extra-cutaneous dissemination, with an average rate of 5-year survival varying from 89 to 96%. Cutaneous lymphoma should be always become one of considered diagnosed of skin red nodules even it is rare.
Topics: Humans; Female; Middle Aged; Skin Neoplasms; Skin; Melanoma; Diagnosis, Differential
PubMed: 37524604
DOI: No ID Found